Abstract

Objective: Syringomyelia associated with osteogenesis
imperfecta and basilar impression is a rarely seen pathology.
We herein report our management experiences of patients with
this rare condition.
Methods: We overlook 4 patients with osteogenesis imperfecta,
basilar impression and syringohydromyelia. All patients were
symptomatic at presentation. One patient received a syringosubarachnoid
shunt at the craniocervical junction and 2 patients
underwent cranio-cervical decompression with duraplasty. One
patient was followed conservatively. Follow up periods of these
patients ranged up to 14 years.
Results: Two patients that received a cranio-cervical decompression
showed complete remission of clinical symptoms through the followup
period. One patient who was treated by a syringo-subarachnoid
microcatheter shunt had early regression of spinal cord symptoms,
but died 6 weeks after surgery. One conservatively treated patient
remained neurologically unchanged and therefore surgery was not
advocated.
Conclusions: Cranio-cervical decompression and duraplasty is a
treatment option for symptomatic patients with syringohydromyelia
and basilar impression accompanying osteogenesis imperfecta
resulting in acceptable perioperative morbidity.